Untitled

• The lecture will cover health promotion, restoration, and maintenance for families with hematologic and neoplastic dysfunctions in children.

Hematologic Disorders

• These include lead poisoning, sickle cell anemia, and hemophilia.

Lead Poisoning

• About 310,000 US children ages 1-5 have elevated blood lead levels, which can accumulate over months and years and cause serious health problems.
• Children absorb up to 70% of lead, whereas adults absorb about 20%.
• Lead poisoning is often undetected, as there can be no obvious symptoms.
• It can lead to learning disabilities, behavioral problems, malformed bones, and slow growth.
• Very high lead levels can cause seizures, coma, and death.
• Lead exposure typically occurs by hand-to-mouth contact or through lead dust in the environment.
• Some cultures use medicinal remedies that contain lead.
• Lead in paint was banned in 1978.
• Clinical manifestations interfere with the hematologic, renal, and neurologic systems.
• Other clinical manifestations are nausea, abdominal pain, anorexia, anemia, glycosuria, proteinuria, ketonuria, decreased Vitamin D, distractibility, hyperactivity, intellectual deficits, and lead encephalopathy, which is the most serious effect.
• Blood lead levels have specific actions of therapy, depending on the level.
• Chelating agents bind with lead to remove it but does not alter the effects of lead encephalopathy.
• Chelating agents are given over several courses of treatment.
• Monitor renal, hepatic, and hematological parameters due to side effects of treatment.

Sickle Cell Anemia

• Sickle cell anemia is a genetic disease.
• A chronic disease occurring in 2000 newborns yearly in the US.
• It affects one out of every 500 Black or African American births.
• Affects one out of every 36,000 Hispanic American births.
• Commonly seen in people of Mediterranean, Middle Eastern, and Indian descent.
• There is a 40% incidence in Africans native to West Africa.
• It is an autosomal recessive disorder.
• If both parents have the trait, there is a 25% chance of producing a child with SCA.
• 25% will have normal hemoglobin.
• 50% chance of producing a child with the trait and will be a carrier.
• Sickle cell disease equals predominantly having Hgb SS.
• Newborns with SCA are asymptomatic until later in infancy.
• Presence of fetal hemoglobin (60-80% of Hgb F) protects the infant.
• Studies have revealed that Hgb F usually disappears from red blood of infants after about 6 months.
• Clinical manifestations occur when normal adult hemoglobin (Hgb A) is partly or completely replaced by abnormal sickle hemoglobin (Hgb S).
• The rigid sickle-shaped RBCs become entangled with one another and cause the microcirculation and increase destruction of RBCs, causing a decrease in oxygen-carrying capacity.
• Every organ can be effected by vaso-occlusion causing local hypoxia, tissue ischemia and cellular death.
• Sickled cells increase the viscosity in the area where they are involved in the microcirculation.
• Clinical signs of sickle cell crisis can be general.
• Symptoms vary greatly in severity and frequency.
• Increases the risk for sepsis, chronic anemia
• Vasoocclusive crisis may occur, Ischemia, causing mild to severe pain in the involved area.
• Sequestration crisis results from pooling of large amounts of blood in the spleen leading to circulatory collapse due to blood volume.
• Aplastic anemia occurs when there is decreased RBC production, -> profound anemia
• Hyperhemolytic crisis occurs when there is increased RBC destruction, -> anemia, jaundice & reticulocytosis
• Acute Chest Syndrome is clinically similar to pneumonia, -> pulmonary infiltrate, chest pain, fever, cough, tachypnea, wheezing & hypoxia
• Cerebrovascular accident occurs due to occluded blood vessels in the brain causing strokes and variable degrees of neurologic impairment.
• Mandatory newborn screening is done in all 50 states using the Sickle-turbidity fingerstick test (Sickledex), with results in 3 minutes.
• If the Sickledex test is positive, Hgb electrophoresis is necessary to determine if the child has trait or the disease itself, confirming the diagnosis.
• Prevention of sickling as a goal of therapy, as well as treating medical emergencies for crises
• Supportive care includes rest to minimize oxygen use, hydration to reduce blood viscosity, and electrolyte replacement therapy for hypoxia and metabolic acidosis.
• Analgesics, though usually undermedicated, and Tylenol or Motrin for fever if not contradicted, and do not use cooling blankets.
• Anemia can be treated with blood transfusions.
• Use antibiotics to treat infection and oral penicillin prophylactic.
• Vaccines for pneumococcal, meningococcal, and flu.
• Oxygen to prevent severe hypoxia.
• Partial Exchange RBC transfusion to get rid of the bad and in with the good.
• Splenectomy for life-threatening splenic sequestration.
• Mild to moderate pain can be treated with ibuprofen, ketorolac (Toradol), acetaminophen, or codeine.
• Severe pain is treated with narcotics ATC (PO or IV), in Crisis-IV route.
• PCA administration gives patient control
• Meperidine (Demerol), a CNS stimulant, which will produce anxiety, tremors & seizures with repetitive dosing--avoid administration.

Hemophilia

• Hemophilia is a deficiency of coagulation factor (proteins) in the blood.
• It is an X-linked hereditary autosomal recessive disorder.
• Affected males inherit the trait through carrier mothers.
• Affects 1 in 5000 males.
• Factor VIII deficiency (hemophilia A) accounts for 80% of all hemophilia cases.
• Other types are Factor IX deficiency (hemophilia B) and Factor XI deficiency (hemophilia C).
• Females with one normal X chromosome and one mutated X chromosome generally do not manifest the disorder but are carriers and can pass on hemophilia.
• On the other hand, since a male only has one X chromosome, if it is mutated he has no normal copy and will develop the disorder.
• Diagnosis consists of history of bleeding episodes, evidence of x-linked inheritance, as well as PTT labs
• Lab assay procedure to detect deficiency type
• DNA testing can detect carriers in families in which female offspring may have inherited the trait
• Treatment is Factor VIII injection with Antihemophilic factor (Bioclate).
• Clinical manifestations include prolonged bleeding to affected areas, traumatic hemorrhage, excessive bruising, subcutaneous and intramuscular hemorrhages, Hemarthrosis with knees, ankles & elbows, Hematomas, and Hematuria.

Neoplastic Disorders

• These disorders include Rhabdomyosarcoma, Retinoblastoma, Wilms Tumor, and Neuroblastoma.

Rhabdomyosarcoma

• Rhabdomyosarcoma is a soft tissue tumor arising from cells that would ordinarily form striated muscle.
• Tumors are commonly located in the head, neck, GU tract, and extremities.
• Tumors are highly malignant and cells spread through the venous or lymphatic system.
• Age 2-10 years, often times presents with an asymptomatic mass
• Definitive diagnosis requires primary tumor biopsy
• CT, MRI, bone marrow aspiration, bone scan and skeletal survey.
• There are 4 stages.
• Symptoms determined by the site involved.
• Surgical resection of the tumor.
• The treatment is determined by the site and size of the tumor, with the addition of radiation and chemotherapy.
• Complications consist of a highly malignant tumor with metastasis commonly to the lungs, bone, bone marrow, and the CNS.

Retinoblastoma

• Retinoblastoma is a highly malignant tumor of the retina.
• Diagnosed by age 3 with a survival rate of 90%.
• In nonhereditary cases, the mutated cell occurs early in life and only in only one eye.
• In hereditary cases, all the cells in the body, including those in both eyes, can have a mutated defective RB1gene
• The condition can develop either after conception while in the womb (75%) or can be inherited from one parent (25%, autosomal dominant)

Wilms Tumor

• It is the most common malignant renal and intraabdominal tumors.
• Peak age is 2-3 years, affecting boys slightly more than girls.
• 1-2.5% of cases have a familial origin.
• Usually unilateral with 6% bilateral involvement.
• May metastasize, commonly to perirenal tissue, liver, diaphragm, lungs, abdominal muscles, and lymph nodes, but metastasis is rare.
• Diagnostics are thorough history and physical exam.
• Tests include X-rays, CTs, hematologic studies, and bone marrow aspirations to rule out metastasis.
• Symptoms can involve weight loss, enlarged liver/spleen, anemia, lymphadenopathy, abdominal mass and/or pain, fatigue, malaise, hypertension, vomiting, anorexia, and/or constipation.
• In cases with lung metastasis, dyspnea, SOB, and chest pain may be experienced.
• Radiation and chemo may be administered before or after surgery, nephrectomy.
• Care must be taken to prevent the spread of cancer cells during surgery after diagnosis.
• Nursing consideration: Do not palpate the abdomen.
• Post-op care is the same as with abdominal surgery.
• Teach to avoid contact sports and look for S & S of GU dysfunction.
• Stages I & II of Wilm's Tumor have a 90% chance of cure with multimodal therapy.
• Wilm tumor may recur, especially in the lungs.
• Both chemo & radiation can induce secondary malignancies, usually in the areas that have been irradiated

Neuroblastoma

• It is the most common malignant extracranial solid tumor of childhood.
• The majority are diagnosed before 5 yrs of age.
• Majority of tumors develop in the abdomen along the adrenal gland or retroperitoneal sympathetic chain
• Metastasis may occur in the head, neck, chest, and pelvis
• It is referred to as a "Silent Tumor", sometimes with a diagnosis after metastasis.
• Survival rates are about 40-90% depending on staging.
• Favorable diagnosis if younger than 12 months old and/or the presence of stage I disease.
• For diagnostics include locating primary tumor sites.
• A system assessment is performed based on the location of the tumor.
• Radiologic testing is needed for definitive diagnosis.
• Treatment includes clinical staging to establish plan, surgery, and radiation & chemotherapy.